Specialized equipment for spinal muscular atrophy

Last updated Feb. 6, 2023, by Susie Strachan
Fact-checked by Patricia Silva, PhD

People with spinal muscular atrophy (SMA) often need specialized equipment to help support them in daily activities, assist with mobility, and aid with breathing, coughing, and swallowing issues.

SMA is divided into five main types — 0, 1, 2, 3, and 4 —  and equipment needs depend on the severity of the condition. Type 0 is the most severe form of the disease, with an onset before birth, while type 4 is its mildest form, occurring in adulthood.

There is a range of different specialized equipment options available to assist those living with SMA, including walking aids, such as braces, grab bars, and electric wheelchairs, bath chairs, breathing and feeding equipment, and car beds for traveling.

Visiting a practitioner who has experience working with SMA patients can help individuals decide which specialized equipment is best suited to their daily life.

What symptoms of SMA require specialized equipment?

SMA is a genetic condition causing progressive muscle weakness and wasting due to mutations in the SMN1 gene. If a person shows common SMA symptoms and/or has a family history of the disease, tests should be conducted to enable a diagnosis. There are treatments that can help manage the condition by targeting the underlying cause of the disease.

The most common symptoms include:

• poor muscle tone and control, which impair patients’ motor development and/or future mobility
• movement issues, most notably never acquiring or gradually losing the ability to sit, walk, or move
• breathing difficulties, including problems with feeding and swallowing
• scoliosis, a sideways curvature of the spine, associated with weakness in the muscles supporting the core area
• increased risk of lung infections, including aspiration pneumonia, which is a lung infection that develops due to food or liquid being aspirated into the lungs.

Mobility and home life

Mobility can be a challenge for people with SMA. Specialized equipment, such as adaptive strollers and wheelchairs, can help optimize their care and maximize their independence. Life at home may require assistive equipment for tasks such as sitting up, getting dressed, eating, and managing stairs.

Adaptive strollers

Also called medical strollers, adaptive strollers allow a child to be supported and positioned comfortably. They are usually lighter and more portable than wheelchairs, and can easily be loaded in and out of a vehicle. Accessories also can help with comfortable positioning and posture, or with carrying medical or other equipment. Adaptive strollers are often used until a child is big enough for a wheelchair. Some examples include:

• Convaid offers a wide range of convenient, customized, and compact folding strollers or wheelchairs
• The Maclaren major special needs stroller will carry a large child, and fold to the size of a big umbrella-like stroller. Its manufacturer recommends it for younger children with mild disabilities.
• Special Tomato EIO push chairs, also by Maclaren, are designed for young children up to preadolescence, and are easy to adapt to a child’s needs and to transport.
• Thomashilfen offers a number of easy to use and adjustable strollers.

Wheelchairs

Wheelchairs are mobile chairs, either manual or powered. Manual chairs can be manipulated by their user or pushed by a caregiver. Power chairs are electronically controlled with a joystick that is usually placed on the side of the dominant hand, and are an alternative for people who are not able to manipulate a chair with their arms, allowing for greater independence. Children as young as 18 months old can learn how to use power chairs.

• Convaid has lightweight and foldable wheelchairs with various accessories for customization according to a patient’s needs.
• Troy Technologies‘ wheelchairs are foldable, much like an umbrella-style folding stroller, for easy storage and transport. Many are designed for young adults with special needs.

Walkers

People with SMA can use a walker to assist with mobility and maintain independence. Using a walker can be combined with gait training by a therapist, including work on increasing strength, balance, coordination, and endurance.

Adaptive bicycles

Older children and adults with SMA may enjoy adaptive bikes, which support independence of mobility and play with family and peers. Using a bike provides exercise, improves balance, muscle strength and posture, and builds cardiovascular capacity. Some can be pedaled by hand, while tandem bicycles allow an adult to pedal while the child rides in front, behind, or beside. Bikes can be outfitted with electric motors to assist the rider.

Hybrid assistive limb (HAL)

Although still in the experimental stage, a wearable walking aid called a hybrid assistive limb (HAL) is being evaluated to improve ambulatory function in those living with SMA. A 2021 study in 24 patients with neuromuscular disorders, including five with SMA, who were unable to walk for 10 meters independently, showed that cybernetic treatment with HAL significantly improved the distance walked, when compared to using only a hoist. The subjects of this study had neither respiratory failure nor rapid deterioration in gait.

Car beds

Younger children may not be able to breathe properly in a standard car seat. Car beds allow children to recline or lie flat, which aids in breathing

Bath chairs

These chairs ensure bathing is safe and secure for children and adults with special needs. They typically can be adjusted to different positions depending on the person’s requirements, and the height can also be adjusted according to the needs of the parent or caregiver.

Feeder seats

Specially designed feeder seats exist for all ages. These are typically soft, foam positioning seats that are fairly easy to use. They can be a good alternative to a wheelchair or stander.

Feeding tubes

Tube feeding is a method that allows a person with SMA to be fed through a tube leading directly into their stomach or small intestine.

People with SMA may have trouble digesting foods, difficulty swallowing, and may struggle with weight management. Metabolizing fat in foods can be difficult, and people with SMA can be at risk of becoming overweight.

There are feeding tubes used for both the short and long term.

Short-term feeding tubes include nasogastric tubes and nasojejunal tubes.

• The nasogastric tube is inserted through the nose and all the way to the stomach.
• The nasojejunal tube is inserted through the nose and all the way to the small intestine.

Long-term feeding tubes include gastrostomy tubes and percutaneous gastrostomy (PEG) tubes. Both types are inserted in hospital, with the patient under anesthesia.

• Gastrostomy tubes are inserted through the abdominal wall and into the stomach.
• A percutaneous endoscopic gastrostomy (PEG) is a procedure to place a feeding tube, using endoscopic surgery to make an opening in the upper abdomen and connect the tube to the stomach.

There are risks associated with both types of feeding tubes, including pain at the tube site, infection, constipation, nausea, and vomiting. The stomach contents also may leak and the tubes themselves may become clogged or damaged.

Depending on the type of feeding tube, a doctor or nutritionist will write a prescription for the feedings (known as formulas). The most common tube feeding methods include bolus feeding, intermittent drip feeding, and continuous feeding.

Tube feeding can have negative psychological effects on a child with SMA and their parents. It’s important parents discuss alternatives, consequences, and planning for the long term with their healthcare providers.

Respiratory

Support is of utmost importance, especially in aiding younger children with SMA to avoid a tracheostomy, lung collapse, or hospitalization over other respiratory issues. At home, both a bi-level positive pressure (BiPAP) machine and cough assist machine are used to maintain good respiratory health.

BiPAP machine

A BiPAP is a noninvasive ventilation breathing support device. Worn as a mask over the nose and mouth, or as a tube to the nose, it can make it easier for an SMA patient to breathe by changing the pressure of air during inhalation and exhalation, as the machine provides two levels of positive airway pressure: higher pressure and an increased volume of air when breathing in, and lower pressure, automatically adjusted, when breathing out.

Cough assist machine

Weakness of the chest muscles can make it difficult to clear mucus and other secretions in the respiratory tract. A cough assist machine can help ensure the airways stay clear. It is adaptable, and can be used as a mouthpiece or face mask. This device uses a positive air pressure that rapidly changes to negative pressure, assisting the patient with coughing. When the child breathes in, the machine provides air (positive pressure) to expand the lungs; when breathing out, the machine rapidly creates a sucking force (negative pressure), helping to remove airway secretions.

Orthopedic

Due to the difficulty maintaining good body alignment, people with SMA may be at risk for orthopedic problems related to bone, muscles, and ligaments. Devices such as braces and standers may be used to support different parts of the body.

Braces

One of the most common concerns in SMA is spinal alignment. Scoliosis is an abnormal spine curvature that occurs due to weakness in muscles that normally support the spine. Besides affecting comfort, the ability to balance when sitting and standing, and arm function, scoliosis can also affect breathing and cause the patient to require surgical treatment.

Braces can help people with SMA achieve a more upright position, improving function, independence, comfort, and quality of life. Braces come in three different types:

• Ankle-foot orthoses, which are externally applied and intend to control the position and motion of the ankle, compensate for weakness, and correct deformities. They can be used to give support to weak limbs or to position a limb with contracted muscles into a normal position.
• Knee-ankle-foot orthoses are designed to provide support and proper joint alignment to the knee, foot, and ankle. They are used to help or replace weak muscles and protect the foot and lower limb.
• Thoraco-lumbo-sacral orthosis are back braces that wrap beneath the arms and around the thorax, lower back, and hips to help cast the spine in a straighter position.

Standers

Some people with SMA are unable to stand independently. Standers can help by promoting bone and muscle strength because users bear weight in their legs. They also help with digestion, circulation, and breathing while enhancing social interactions, improving self-confidence, and promoting overall mental health. Available standers include:

• EasyStand has a wide range of standers developed to help patients starting at age 9 months participate in supervised standing programs.
• Jenx standing frames were designed to support people with special needs of all ages. Their products take into account the importance of standing for a better development by stabilizing joints.
• Kaye manufactures and sells mobility aids, adaptive positioning equipment, and therapy products for infants, children, and young adults with special needs.
• Leckey standers are designed to be functional, attractive, and robust for people with special needs from birth to adulthood.
• Rifton standers are adjustable to meet children’s growth capacity.
• R82 standers are made for children and young adults to provide support while standing.

Pulse oximeter

A pulse oximeter is a portable, noninvasive device used to check blood-oxygen levels, by measuring the amount of oxygen in the blood (pulse oximetry).

A child with SMA may need to be monitored for respiratory distress. A pulse oximeter can be used to measure the level of oxygen saturation in their blood. It can monitor how efficiently oxygen is being carried to their extremities including legs and arms.

The machine consists of a light small clamp or a Band Aid-like device that is wrapped on a finger or toe. The equipment alerts to problems (e.g., low oxygen levels) or if help is needed with coughing.

Healthcare providers can use remote sensing devices including a pulse oximeter for remote monitoring of patients. Overall perception of the device is positive, but there can be difficulty getting an accurate reading.

Pulse oximeters, provided through companies including SMA Medical Supply, Flaghouse, or Philips Respironics, include:

• Masimo Radical-5 handheld pulse oximeter is a lightweight handheld device with a battery life of up to 48 hours. It can be used either for spot checks or continuous monitoring. It has sensitivity options, audio and visual alarms for high/low saturation, pulse rate, sensor off and low-battery warnings. It requires a physician’s prescription.
• Masimo Radical-8 is compact and lightweight with an extended battery life for easy transportation, and more than eight hours of battery life when fully charged. The large LED display allows easy reading. It requires a physician’s prescription.
• EKHO P-900 Finger Pulse Oximeter is a small, portable and electrocardiogram (EKG)-accurate device. It displays the pulse rate, real-time pulse bar, and has an auto on/off oxygen saturation.
• GO2 Pulse Oximeter is a portable device that offers accurate and reliable oxygen saturation and heart rate monitoring information. It requires one AAA battery.
• 920M Handled Oximeter is a small, easy-to-use handheld unit for the home. It has 18 hours of memory capability.
• 920M Plus Handheld Oximeter can record up to 72 hours of data and allows for longer oximetry studies. It has a small, light, and portable design, and easy-to-read display. It offers an optional rechargeable battery system.
• WristOx2 Wrist-worn Pulse Oximeter is accurate and highly portable, being worn on the wrist and using standard batteries. It is ideal for overnight oximetry studies, remote wireless monitoring, and ambulatory monitoring.
• Masimo LNCS Probes are used with devices to offer accurate detection of true alarms while minimizing false alarms. The sensors can connect to devices with conventional technology, allowing ease of use with a variety of monitors.

SMA News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.